Cardiac MRI Predictors of Arrhythmic Sudden Cardiac Events in Patients With Fontan Circulation

Natasha K Wolfe; Mary D Schiff; Laura J Olivieri; Adam B Christopher; Mark Fogel; Timothy C Slesnick; Rajesh Krishnamurthy; Vivek Muthurangu; Adam L Dorfman; Christopher Z Lam; Justin Weigand; Joshua D Robinson; Rahul H Rathod; Tarek Alsaied; FORCE Investigators

doi:10.1016/j.jacc.2024.08.063

Cardiac MRI Predictors of Arrhythmic Sudden Cardiac Events in Patients With Fontan Circulation

J Am Coll Cardiol. 2024 Oct 14:S0735-1097(24)08394-3. doi: 10.1016/j.jacc.2024.08.063. Online ahead of print.

Authors

Collaborators

FORCE Investigators:
Yue-Hin Loke¹³, Kenan Stern¹⁴, Jonathan H Soslow¹⁵, Thomas P Graham Jr¹⁵, Manish Aggarwal¹⁶, Tiffanie Johnson¹⁷, Markus Renno¹⁸, Amee Shah¹⁹, Michael Quail²⁰, Shelby Kutty²¹, Helen Taussig²¹, Ruchira Garg²², Jeremy Steele²³, Pierangelo Renella²⁴, Madhuradhar Chegondi²⁵, Aswathy Vaikom House²⁶, Alison Marsden²⁷, Francesca Raimondi²⁸, Matthew Files²⁹

Affiliations

¹ Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Electronic address: wolfena@upmc.edu.
² Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
³ Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
⁴ Department of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
⁵ Department of Radiology, Nationwide Children's Hospital, Columbus, Ohio, USA.
⁶ UCL Centre for Cardiovascular Imaging, Institute of Cardiovascular Science, University College London, London, United Kingdom.
⁷ Department of Pediatrics, University of Michigan Medical School, Ann Arbor, Michigan, USA.
⁸ Department of Diagnostic and Interventional Radiology, Hospital for Sick Children and Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada.
⁹ Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.
¹⁰ Department of Pediatrics, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
¹¹ Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
¹² Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Electronic address: https://twitter.com/nkwcardiomd.
¹³ Division of Pediatric Cardiology, George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
¹⁴ Division of Pediatric Cardiology, Icahn School of Medicine at Mount Sinai, Kravis Children's Hospital, New York, NY.
¹⁵ Division of Pediatric Cardiology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA.
¹⁶ Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO, USA.
¹⁷ Department of Pediatrics, Indiana University School of Medicine, Riley Children's Hospital, Indianapolis, IN, USA.
¹⁸ Department of Pediatrics, University of Arkansas, Arkansas Children's Hospital, Little Rock, AR, USA.
¹⁹ Division of Pediatric Cardiology, Department of Pediatrics, Columbia University Irving Medical Center, New York, NY, USA.
²⁰ Division of Pediatric Cardiology, Department of Pediatrics, University College London, Great Ormond Street Children's Hospital, London, UK.
²¹ Division of Pediatric Cardiology, Department of Pediatrics, Johns Hopkins University, Johns Hopkins Children's Hospital, Baltimore, MD, USA.
²² Division of Pediatric Cardiology, Department of Pediatrics, Cedars-Sinai Children's Hospital, Los Angeles, CA, USA.
²³ Division of Pediatric Cardiology, Department of Pediatrics, School of Medicine, Yale University, New Haven, CT, USA.
²⁴ Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Orange County, Orange, CA, USA.
²⁵ Division of Pediatric Cardiology, Department of Pediatrics, University of Iowa, Iowa City, IA, USA.
²⁶ Division of Pediatric Cardiology, Department of Pediatrics, University of Oklahoma, Oklahoma City, OK, USA.
²⁷ Division of Pediatric Cardiology, Department of Pediatrics and Bioengineering, Stanford University, Palo Alto, CA, USA.
²⁸ Division of Pediatric Cardiology, Papa Giovanni XXIII Hospital, Bergamo, Italy.
²⁹ Division of Pediatric Cardiology, University of Washington / Seattle Children's Hospital.

PMID: 39453360
DOI: 10.1016/j.jacc.2024.08.063

Abstract

Background: Among patients with congenital heart disease, those with single ventricles have the highest risk of early mortality. Sudden cardiac death is an important cause of death in this population. Understanding the risk factors for sudden cardiac events (SCE) in Fontan patients could improve prediction and prevention.

Objectives: The goal of this study was to determine the prevalence of SCE and risk factors for SCE in the Fontan population.

Methods: The Fontan Outcomes Registry Using CMR Examinations (FORCE) is an international registry collecting clinical and imaging data on Fontan patients. SCE was defined as: 1) cardiac arrest from a shockable rhythm; 2) need for emergent cardioversion/defibrillation; or 3) documented sustained ventricular tachycardia. Univariate and multivariate Cox proportional hazards regression models estimated hazard ratios for predictors of SCE.

Results: Our sample included 3,132 patients (41% female). The median age at first cardiac magnetic resonance was 14.6 years. SCE was experienced by 3.5% (n = 109) over a median follow-up time of 4.00 years. Of the 109 patients with SCE, 39 (36%) died. On multivariable analysis, NYHA functional class >II (HR: 4.91; P < 0.0001), history of protein-losing enteropathy/plastic bronchitis (HR: 2.37; P = 0.0082), single-ventricle end-diastolic volume index >104 mL/m² (HR: 3.15; P < 0.0001), and ejection fraction <50% (HR: 1.73; P = 0.0437) were associated with SCE. Kaplan-Meier analysis demonstrated that in patients with none of the above risk factors, the 4-year freedom from SCE was 99.5%.

Conclusions: SCE occurred in 3.5% of the study population, and one-third of patients who experienced SCE died. Mild ventricular dysfunction and dilatation by cardiac magnetic resonance, NYHA functional class, and history of protein-losing enteropathy/plastic bronchitis were associated with SCE.

Keywords: Fontan; arrhythmia; cardiac magnetic resonance; cardioversion; congenital heart disease; sudden death.